CASE REPORTS
Burkitt Non-Hodgkin Lymphoma in childhood
José Henrique Barreto
Burkitt's Non-Hodgkin Lymphoma (B-NHL) has a high incidence at Equatorial Africa. It is endemic and associated with the Epstein-Barr Virus (EBV) infection. AIDS pandemic has increased the incidence of this neoplasia that has a close relationship with the EBV infection. In New Guinea the Burkitt's Lymphoma is highly associated with EBV and this association seems to be similar to those found in some regions of Latin America, which has similar socioeconomics and climatic conditions. An example of that is the Bahia State (and other regions of the Northeast) that shows a higher frequency of Burkitt's lymphoma associated to EBV than Southeast Brazilian states and Argentina. In the Brazilian Northeastern Region Burkitt's lymphoma seems to have an intermediate frequency between sporadic and endemic types. The high incidence of abdominal presentation of Burkitt's lymphoma (as opposed to the jaws presentation, in North of Africa) suggests common mechanisms that still need to be elucidated. The high incidence of Burkitt's lymphoma in children is similar to those found in other reports. Apparently a better immunologic control as the child grows decrease the number of EBV infected cells, target of neoplastic transformation.
The treatment in the majority of the Brazilian centers is in accordance to the Brazilian Cooperative Group for Childhood Lymphomas Treatment, protocol NHL 2000. In prognostic terms, is expected that clinical remission of 90% of low risk B cells NHL and 70% to 80% for the high risk B cells NHL. From January 2000 to September 2004, 76 NHL patients between 0 and 19 years old were evaluated. The mean age was 8,1 years (range 2-16yrs). There were 49 male and 27 female patients. 14 patients were white and 11 black 51 mixed. 39.5% of the patients came from the rural areas. 44 (57.8%) have B-NHL, 17 (22.4%) T lymphomas, 9 (11.4%) large-cells anaplastic lymphomas, 5 (6.6%) large-cells diffuse lymphomas and 1 (1.3) nasal angiocentric lymphoma. The most frequent symptoms were abdominal pain (23), followed by increased abdominal volume (14) and cervical tumors (14). Other related symptoms were abdominal tumor (7), dispnea (6), cough (3) and vomiting (3). There were other less frequent symptoms related. The most affected region was abdomen (45; 59.2%), cervical (11; 14.5%), mediastine (13; 17.1%). Others were: nose, inguinal, mallar, oropharynx, paravertebral and testicle (1 case each). The presence of bone involvement was observed in 23.7% of the patients and 3.9% presented CNS involvement. Time to diagnosis ranged from 1 to 210 days (mean = 47.1 days). The mean LDH was 2,014 U/mL (range 91U to 36,599U). 51 patients had immunephenotyping, 23 confirmed B (Burkitt's) NHL, 13 T NHL patients, 2 T angiocentric NHL patients (one nasal e one testicular), 2 mediastinal diffuse large cell NHL patients (B), 4 abdominal diffuse large cell NHL patients (B), 7 large cell anaplastic lymphoma patients (T). 51 (67.1%) patients are alive and with no evidence of disease, 22 (28.9%) were dead, 2 (2.6%) alive (but with short follow-up) and 1 patient was lost of follow-up. The causes of deaths were: progressive disease (13; 59.1%), sepsis (7; 31.8%) and deep venous thrombosis and cardiogenic shock (1; 4.5% each).
Publication Dates
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Publication in this collection
06 Jan 2006 -
Date of issue
Oct 2005
